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By locating itself on the face, it can transform the greatest beauty into an unrecognizable creature in a few minutes, scaring the patient and his surroundings. However, aesthetic considerations are often just the beginning of the problems. Swelling involving the larynx is a direct threat to life and may end tragically. Chronic forms, on the other hand, are a real diagnostic and therapeutic challenge.
Angioneurotic edema, also known as Quincke’s edema, is a type of swelling that occurs within the body’s subcutaneous and submucosal tissue. The formation of this type of edema results from a sudden increase in the permeability of blood vessels in the skin and mucous membranes. The plasma emerging from “holes” and penetrating into the intercellular space causes edema. This swelling is usually limited, with clearly marked borders, tight, non-dense (no pits remain after pressing). The lesions are often located asymmetrically, mainly in the area of the face – they affect the lips, eyelids, tongue, as well as hands, feet and sometimes genitals. Although they can take a very severe, deforming form of the face, they usually do not cause significant complaints in the patient. There may be only slight local soreness and there may be a problem with the tongue getting into your mouth.
However, it should be remembered that the edema may also affect the upper respiratory tract. The developing laryngeal edema prevents air from flowing freely to the lungs. There is increasing shortness of breath, hoarseness which over time turns into silence, problems with swallowing, drooling and a characteristic whistling (stridor) when inhaling air. The condition requires immediate treatment as failure to address promptly may result in suffocation.
Edema of the gastrointestinal mucosa is much more difficult to diagnose, especially often in congenital forms of the disease. The symptoms of the swelling of the intestinal mucosa are very nonspecific and resemble those of other abdominal ailments. The patient complains of severe abdominal pain, while imaging tests (X-ray / ultrasound of the abdomen) show distended, swollen intestinal loops.
The diagnosis of angioedema is determined by the presence of very characteristic symptoms of the disease, with edema localized in typical places and with a characteristic appearance. However, a variety of mechanisms may be responsible for its creation. Thus, the following forms of Quincke’s edema are mentioned: allergic, pseudoallergic, non-allergic and idiopathic (with an undetermined cause).
The allergic form of angioedema is associated with sensitization. Symptoms of edema appear within minutes to two hours of contact with the substance causing the allergy (e.g. medicine, food, insect venom, latex). Even in half of the patients, urticaria is a comorbid symptom. Additional tests confirm sensitization. On the other hand, the increased permeability of blood vessels results, among others, from from a sudden release of histamine, a chemical contained in white blood cells (mast cells). Histamine getting into the blood causes, among others vasodilation. This reaction is mediated by IgE antibodies.
However, it is known that histamine can be released from mast cells in the absence of antibodies. This is the case with angioedema that occurs after contact with non-steroidal anti-inflammatory drugs or contrast agents. The most common cause in this group is aspirin.
There are also forms of Quincke’s edema that have nothing to do with allergic reactions, and increased vascular permeability is due to an entirely different disorder. Here, by far the most common cause of the so-called Acquired angioedema, which is also the main reason for patients to come to the emergency room, is the use of antihypertensive drugs from the group of angiotensin converting enzyme inhibitors, the so-called ACE inhibitors. Angioedema in the group of patients treated with this type of drugs occurs in even one in five hundred patients and occurs most often within the first week of starting the drug. However, about a third of cases occur after months and years of treatment. It is also known that the acquired form of edema may accompany other chronic diseases from the field of systemic diseases of connective tissue, as well as hematological neoplasms (leukemias, lymphomas, myelomas).
Edema resulting from congenital disorders is also non-allergic. The so-called hereditary angioedema is a group of conditions in which edema is caused by inherited disorders of the plasma proteins that regulate the permeability of blood vessels. The diagnosis of this form of the disease requires specialized examinations and tracing the history of the family history of the disease. Symptoms of the disease are caused by various types of stressful situations for the body, such as surgery, dental or emotional stress, as well as alcohol. Type III of the disease is particularly predilection for women, and hormonal factors also play a role in the mechanisms of the disease. Symptoms of the disease often become apparent after the implementation of contraceptive hormonal drugs.
Finally, there is also a whole group of angioedema, the causes of which cannot be determined and are therefore called idiopathic. However, before such a diagnosis is made, the presence of all the above-mentioned disorders should be ruled out. The medical observation also shows that the factors predisposing to the appearance of angioedema are various types of infections (bacterial, including H. pylori, viral and parasitic).
The treatment of the disease depends largely on its causes. The most important thing is to assess the occurrence of respiratory tract involvement and the impending laryngeal edema. Then the most important goal of treatment is to keep the airways open. The treatment of choice is adrenaline. If necessary, it is important to make a quick decision about intubation, as it becomes impossible when the larynx is completely swollen. Then a tracheotomy becomes necessary. In allergic forms, antihistamines administered in non-standard high doses and glucocorticosteroids are also used. In the acquired form associated with the use of antihypertensive drugs, changing the drug controlling blood pressure is also of key importance. In the case of idiopathic and allergic edema, medications that affect the functions of the immune system can be helpful. In congenital forms of the disease, the optimal procedure is to supplement the deficiencies of proteins responsible for the symptoms.